What is Primary Biliary Cirrhosis (PBC)?

PBC is a slowly progressive bile duct disease, which results from chronic inflammation of the small intrahepatic bile ducts. It is very variable, very slowly progressive and most patients do not have cirrhosis at diagnosis. For some the inflammation leads to bile duct loss and liver scarring which may, after many years, become extensive and lead to cirrhosis. It is thought to have an autoimmune basis, but we don’t fully understand the triggers (presumably environmental and genetic) that lead to disease in the first place.

How many people are affected by PBC?

It usually affects women between the ages of 40-60 years of age but is seen in some women younger than this, and men can also have the disease. The ratio is 9 women to one man.
A good estimate of its frequency is that 1 in 1000 women over the age of 40 have PBC.

What causes PBC?

At present the cause is unknown. It is known that it is not caused by alcohol.  It is probably a disease triggered by an environmental trigger, in a patient with a subtle genetic predisposition.

What are the manifestations of PBC?

Specific Symptoms
Fatigue - independent of lack of sleep (70-80%)
Pruritus (itching) (50-60%)
Xanthomata - small white bumps under the skin, especially around the eyes.

Nowadays many patients are diagnosed without symptoms (60%)

Complications of Cirrhosis

If liver disease progresses, some of the symptoms that may occur with very advanced disease include:
Ascites (fIuid in the abdomen)
Gastrointestinal bleeding (vomiting blood or passing black bowel movements)
Jaundice  (yellowing of the skin or eyes)
Easy bruising or bleeding

Diseases Associated with PBC

Because PBC is an autoimmune disease it is common for patients or their family members to have other autoimmune diseases. Some of these include:
Thyroid disease
Sicca Syndrome/Sjorgens - dry mouth, dry eyes, dry vagina and dental caries
Raynauds Disease - mottled hands, sensitive to the cold
Rheumatoid Arthritis
Celiac Disease – leads to poor absorption of food as a result of a wheat allergy  

Osteoporosis  (thin bones) is not infrequently seen and sometimes is related to PBC itself, although is common as well in women generally.

Bloodwork

Family doctors usually identify this disease by first noting an increased alkaline phosphatise (enzyme measured in the blood) or an increased GGT (gamma glutamyltranspeptidase) – another enzyme from the liver that can be measured in the blood.

The most characteristic lab finding specific for the disease is AMA (antimitochondrial  antibody) - this antibody is very specific for PBC and is present in 95% of patients.

Other findings can include an increased cholesterol level in the blood.

For most people even if the liver enzymes are elevated, liver function itself is normal.  Bloods tests measuring serum bilirubin, albumin and clotting time can be used to monitor liver function.
 
Liver Biopsy

A small sample of liver is taken using a small needle. The test is safe and performed as a daycase.  It is not always needed to make the diagnosis, or guide treatment, but it does often allow confirmation of the diagnosis and can help estimate the stage of the disease.

How is PBC treated?

Although presently there is no cure for PBC, there are good treatments for the majority.  A treatment using a bile acid, ursodeoxycholic acid, has been shown to significantly decrease the progression of the disease and need for liver transplant.

What is the prognosis of PBC?

For the most part the prognosis is very good, and fewer and fewer people with PBC are needing liver transplant.  This is because the disease is diagnosed early and treated with ursodeoxycholic acid.  For many the disease may remain silent (asymptomatic) and may never become serious. Many can lead normal lives with few symptoms for more than 10 to 20 years. For the small proportion who progress, liver transplant may be needed.  This operation is now very well established and has excellent results.

What lifestyle changes may be helpful for PBC patients?

Patients are encouraged to live a normal lifestyle. Fatigue is a troublesome symptom and pacing daily activities will help and preserve stamina and energy.

Your doctor is likely to recommend you lead a normal healthy life, and aim to do exercise and avoid being overweight.  Smoking is very bad for the liver and patients with PBC who smoke have more scarring.  Alcohol can be drunk in moderation usually.  Other simple advice includes taking calcium and Vitamin D supplements, exercising (walking, running, swimming, etc), skin care, regular dental examinations and artificial tears for dry eyes.

It is wise to consult with your doctor before taking any medication, including painkillers, over the counter drugs and herbal medications. The liver is the body's most important drug metabolizing organ so drugs must be taken with caution.

If I’ve been diagnosed with PBC, what questions should I ask my doctor about PBC?

“How severe is the liver damage?”
“What treatment do you recommend? Will this slow down the progression of the disease?”
“Will any medication be prescribed? What are the side effects?”
“Should I change my diet?”
“Are there any supplements you would suggest that I take?”
“What can be done to relieve my symptoms?”
“If cirrhosis develops, is transplantation going to be needed?”
“Is there a local Hepatologist who looks after patients with PBC and who is specially trained in liver disease I should see?”

It may be advisable to have a family member or friend accompany you, to help take notes and remind you what to ask.

 

 Last modified September 3, 2009